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The objective was to evaluate the long-term results after the construction of a continent catheterizable urinary conduit in adults. We retrospectively reviewed the charts of 41 adults who received a continent catheterizable urinary conduit and extracted indications and complications (29/41 women, average age 32, median follow-up durations 52 months, 48% ≥1 reoperation). Patients who were still using the conduit during this research (n = 32) were sent a questionnaire about their impression of improvement, continence and catheterization problems. 24 patients completed the questionnaire and on average reported a ‘much better’ situation. In conclusion, the construction of a continent catheterizable urinary conduit in adults is associated with a high complication and reoperation rate. Nevertheless, patients perceive improvement compared to before the construction of the stoma.

Objectives: To evaluate the long-term results after the construction of a Continent Catheterizable Urinary Conduit (CCUC) in adults.Methods: This study retrospectively reviewed the charts of 41 adults from two tertiary centers who received a CCUC. The demographics, underlying diseases, indications for a CCUC and outcomes such as the reoperation rate and the occurrence of complications were extracted. The patient reported outcome was measured with the Patient Global Impression of Improvement (PGI-I) scale and four additional questions about continence, leakage and stomal problems. Results: Twenty-nine patients were women. The median age at surgery was 32 years, with a median follow-up of 52 months. Twenty-six patients had a neurogenic bladder. The reoperation rate was 48.8%, with a median of 10.5 months after constructing the CCUC. Superficial stomal stenosis was the most common registered complication (20 times) and stoma revision was the most often performed reoperation (12 times). Twenty-four patients completed the PGI-I; the mean improvement rating was 2 (=much better).Conclusion: The construction of a CCUC in adults is associated with a high complication and reoperation rate. The high reoperation rate is in accordance with the sparse literature. Despite this, patients reported 'much better' on the PGI-I.

Aims To evaluate the long-term outcome of appendicovesicostomies and to present the frequency and timing of complications needing re-intervention. Methods In this retrospective study we included patients in whom an appendicovesicostomy was created at our institution between 1993 and 2011. Patients with a follow-up less than 1 year were excluded. Patient characteristics and conduit-related complications requiring re-intervention were collected. Results One hundred and twenty-eight patients were included with mean age at initial surgery of 10.1 ± 3.9 years. Two thirds of the children had underlying neurogenic disease. The mean follow-up was 10.1 ± 4.8 years. All but one patient continued to use the catheterizable channel. Re-intervention for conduit-related complications was necessary in 32.0% of the patients. A second, third, and fourth re-intervention was required in respectively 10.9%, 2.3%, and 1.6%. The commonest complications were cutaneous/fascial stenosis in 14.8%, stenosis at conduit-bladder level in 9.4%, and stomal incontinence in 6.3% of the patients. The most performed re-interventions were stoma revision (in 16.4% of the patients), conduit revision (10.2%), and dilatation of a stenotic tract (4.7%). 63.3% of the re-interventions was superficial and/or endoscopic. The peak incidence of re-interventions was in the 1st year after conduit construction and decreased yearly. Conclusions Our study gives an overview of patients and their conduits developing from prepubertal children to young adults. During a mean follow-up of 10.1 years, roughly one third of the patients needed a re-intervention. We conclude that an appendicovesicostomy is an effective and durable treatment for whom transurethral clean intermittent catheterization is not feasible. Neurourol. Urodynam. © 2016 Wiley Periodicals, Inc.

Neurogenic lower urinary tract dysfunction (LUTD) in multiple sclerosis (MS) is highly prevalent in adults, but has not previously been described in paediatric MS. A total of 24 consecutive children with newly diagnosed MS were prospectively assessed for bladder and bowel problems early after diagnosis. Five of 24 children (21%) showed LUTD during assessment. One of these patients did not report voiding complaints. This high prevalence of LUTD indicates that all recently diagnosed patients with paediatric MS should be evaluated early in their disease and treated for urinary problems in order to prevent potential damage to the upper urinary tract.

markdownabstractAims: We sought to establish the responsiveness of the Dutch Vancouver Symptom Score for Dysfunctional Elimination Syndrome (VSSDES) and Pediatric urinary incontinence Quality of life (PinQ) questionnaires. Secondary, we evaluated the outcome of urotherapy extended for children with dysfunctional voiding (DV). Methods: This cross-sectional multicenter study was done in one tertiary and two community hospitals. Children with DV were included, also when refractory to previous urotherapeutic treatment. The questionnaires were completed before and after urotherapy. The primary outcome measure was the responsiveness of the Dutch VSDESS and PinQ. Secondary outcome was the initial success (defined by the International Children's Continence Society) of extended urotherapy. Results: Between June 2014 and May 2016, 64 children (median age 7 years, IQR 6-10) received urotherapy (median 18 weeks, IQR 11-28). In contrast to the VSSDES, the PinQ showed good responsiveness. For children and parents, respectively, the area under the ROC-curve was 0.79 (P = 0.01) and 0.72 (P = 0.03) for the PinQ and 0.50 (P = 0.98) and 0.55 (P = 0.62) for the VSSDES. Fifty children received extended urotherapy, 27 had complete, and 14 had partial response. Sixteen children had been refractory to previous treatment; four showed complete, and six showed partial response. Conclusion: The PinQ is able to detect clinically important changes in continence-specific quality of life after treatment. We support the use of the VSSDES questionnaire in addition to the current diagnostics for the diagnosis of DV. Extended urotherapy showed to be a successful treatment for children with DV, also for those who had received previous unsuccessful treatment.

Background:Hypospadias is associated with twinning. The incidence of hypospadias in monochorionic and dichorionic male twins is, however, yet to be determined.Methods:All medical records of monochorionic and dichorionic twins admitted to our neonatal nursery between January 2004 and August 2013 were reviewed for the presence of hypospadias.Results:A total of 350 monochorionic and 303 dichorionic male twins were included in the study. The incidence of hypospadias in monochorionic and dichorionic groups was 4% (14/350) and 1% (3/303) (p= .016) respectively. In 11 of the 15 twin couples, hypospadias occurred in the twin with the lowest birth weight. The rate of hypospadias in twin infants small-for-gestational-age group was 10% (6/60) compared with 2% (11/593) in the appropriate-for-gestational-age group (p= .002). In a multivariate analysis, both monochorionicity and small-for-gestational-age were independently associated with hypospadias, odds ratio 4.1 (95% confidence interval (CI): 1.1–14.7) and 6.1 (95% CI: 2.2–17.2) respectively.Conclusions: The incidence of hypospadias is four-fold higher in monochorionic twins compared with dichorionic twins. Hypospadias is also independently associated with small-for-gestational-age.

Item does not contain fulltext PURPOSE: Bladder dysfunction is common in patients with anorectal malformations and can be congenital or acquired as a consequence of surgery. We investigated the effects of surgical correction of anorectal malformations on bladder function. MATERIALS AND METHODS: The charts of all 341 patients who underwent surgery at our center between 1990 and 2010 were retrospectively analyzed for preoperative and postoperative videourodynamics. A total of 52 patients were eligible for study inclusion. Each assessment was scored according to International Children's Continence Society standards. RESULTS: Urodynamic study indicated normal bladder function preoperatively in 36 patients (69%) and postoperatively in 37 (71%). Median bladder emptying efficiency and relative bladder capacity changed significantly after posterior sagittal anorectoplasty. Bladder function according to International Children's Continence Society standards did not change postoperatively in 43 patients (83%). In 1 of 4 patients with deterioration of bladder function the deterioration could be attributed solely to surgery. Clinical outcome was available in 38 patients and showed complete urinary continence with spontaneous voiding in 24 (63%). Seven of 25 patients (28%) with preoperative videourodynamics indicating normal bladder function demonstrated dysfunctional voiding at latest followup. CONCLUSIONS: Urodynamic and clinical outcomes after anorectal malformation repair are good, with 63% of patients being continent of urine. Urodynamic studies are of limited value in preoperative settings in these patients. Current techniques of reconstructive surgery for anorectal malformations seem to preserve bladder function in the majority of patients.

To report clinical outcomes for laparoscopic pyeloplasty (LP) in children compared with open pyeloplasty (OP) and literature findings.In a prospective study, the outcomes of 57 consecutive transperitoneal LP in children from the age of 3 years were analyzed and compared with a matched historic control group of OP and with series of LP in the literature. Successful result was defined as resolution of symptoms, no conversion or re-operation, improved hydronephrosis, and/or improved renographic drainage.Mean operative time was 177 (SD 50.5)min in the LP group and 108 (SD 25.6)min in the OP group (p0.001). Mean hospital stay was 1.2 (SD 0.46) days in the LP and 6.7 (SD 1.2) days in the OP group. Improvement in renographic drainage was observed more often after LP than after OP (98% vs 83%; p = 0.010). A successful result was reported in 56 (98%) LP and 54 (95%) OP (p = 0.298) patients. Our LP series demonstrates a high success rate compared to literature data.Our LP has a similar success rate and more often improved renographic drainage in comparison to OP. Furthermore, our LP demonstrates a shorter hospital stay and favorable outcomes compared to the literature. We thus regard LP as standard treatment for repair of ureteropelvic junction obstruction in children from the age of 3 years.

To evaluate the long-term outcome of appendicovesicostomies and to present the frequency and timing of complications needing re-intervention.In this retrospective study we included patients in whom an appendicovesicostomy was created at our institution between 1993 and 2011. Patients with a follow-up less than 1 year were excluded. Patient characteristics and conduit-related complications requiring re-intervention were collected.One hundred and twenty-eight patients were included with mean age at initial surgery of 10.1 ± 3.9 years. Two thirds of the children had underlying neurogenic disease. The mean follow-up was 10.1 ± 4.8 years. All but one patient continued to use the catheterizable channel. Re-intervention for conduit-related complications was necessary in 32.0% of the patients. A second, third, and fourth re-intervention was required in respectively 10.9%, 2.3%, and 1.6%. The commonest complications were cutaneous/fascial stenosis in 14.8%, stenosis at conduit-bladder level in 9.4%, and stomal incontinence in 6.3% of the patients. The most performed re-interventions were stoma revision (in 16.4% of the patients), conduit revision (10.2%), and dilatation of a stenotic tract (4.7%). 63.3% of the re-interventions was superficial and/or endoscopic. The peak incidence of re-interventions was in the 1st year after conduit construction and decreased yearly.Our study gives an overview of patients and their conduits developing from prepubertal children to young adults. During a mean follow-up of 10.1 years, roughly one third of the patients needed a re-intervention. We conclude that an appendicovesicostomy is an effective and durable treatment for whom transurethral clean intermittent catheterization is not feasible. Neurourol. Urodynam. 36:1325-1329, 2017. © 2016 Wiley Periodicals, Inc.

We sought to establish the reliability and validity of the Dutch version of the Vancouver Symptom Score for Dysfunctional Elimination Syndrome for children with dysfunctional voiding and their parents.For this cross-sectional multicenter study the Vancouver Symptom Score for Dysfunctional Elimination Syndrome was translated and cross-culturally adapted to Dutch following a standardized process. Patients 16 years or younger with dysfunctional voiding and their parents were recruited at pediatric, pediatric urology and pelvic floor physical therapy outpatient clinics. The reference group consisted of children 6 to 16 years old without dysfunctional voiding and their parents. All groups completed questionnaires. The evaluated measurement properties included discriminative ability, internal consistency, test-retest reliability, interrater agreement, criterion validity using the Pediatric Incontinence Questionnaire and construct validity. A cutoff value for diagnosis of dysfunctional voiding was determined.A total of 50 patients and 60 references and their parents were included in the study. The Vancouver Symptom Score for Dysfunctional Elimination Syndrome showed good discriminative ability. A moderate internal consistency was found (Cronbach alpha 0.37-0.55). Test-retest reliability was moderate to good, and interrater agreement demonstrated good correlation between children and parents (ICC 0.85, 95% CI 0.79-0.89). A weak correlation with the Pediatric Incontinence Questionnaire was found in patients and construct validity was confirmed. Cutoff scores for dysfunctional voiding were 11 and 9 for patients and parents, respectively.The Dutch Vancouver Symptom Score for Dysfunctional Elimination Syndrome displayed moderate to good reliability and validity properties for the patient and parent versions. Use of this instrument in clinical practice will support the assessment of dysfunctional voiding and facilitate international reporting of research results.

Renal candidiasis in neonates and young infants is a potentially life threatening condition. It occurs mainly in neonates and young infants with secondary risk factors and underlying congenital urinar

Purpose The objective was to validate an in vivo model for evaluation of pharmacological effects on bladder function taking the most predominant anticholinergic side effect (hyposalivation) into account. Therefore, two anticholinergic properties (propiverine hydrochloride and tolterodine- l (+)-tartrate) were used to test the in vivo model. Sacral anterior root stimulation (SARS) was performed to induce reproducible and standardized bladder contractions. To evaluate hyposalivation standardised salivavary flow measurements by stimulating the lingual nerve was performed in addition to SARS. Materials and methods 10 male mini pigs were anaesthetised. The carotid artery was cannulated for blood pressure measurement and the jugular vein for administration of propiverine 0.4 mg kg −1 b.w. and tolterodine 0.06 mg kg −1 b.w. For stimulation-induced salivary flow measurements both lingual nerves were exposed and a cuff electrode was placed around the nerves. The bladder was exposed and a cystostomy catheter was inserted to performed cystometrographic measurements during SARS. Results In all experiments, for each animal reproducible intravesical pressure values (pves) and salivary flow rates were elicited during electrostimulation before administration of the drug. Bladder pressure : After administration of propiverine , neurostimulation-induced rise in pves had fallen by 60% from the initial value. After administration of tolterodine pves had fallen by about 50%. After additional administration of atropine pves decreased to about 15% of the initial value for both drugs. Salivation : After propiverine salivary flow had fallen by 61%. Inhibition of salivary flow under tolterodine was about 56%. Additional administration of atropine led in both drugs to a nearly complete blockade of salivation. Heart rate (HR) and blood pressure (BP) : Directly following intravenous administration of both drugs, a short-term and reversible period of mild but significant fluctuations in HR was observed. There was also a slight but non-significant rise in blood pressure. Conclusions This model allows comparative investigations of various drugs with bladder inhibitory properties in terms of acute efficacy and side effects.

OBJECTIVE To establish the value of repeat renal scans to assess the relative renal function after successful paediatric pyeloplasty. PATIENTS AND METHODS Renal scans were assessed from 138 patients after successful pyeloplasty; the relative renal function before and immediately after pyeloplasty were compared, as was split renal function at 3.5 and 5.5 years after surgery. RESULTS With a few exceptions the relative renal function before and after pyeloplasty was stable. Repeat renal scans 3.5 and 5.5 years after surgery also showed stable split renal function, even if the renal function was already diminished. CONCLUSIONS Repeat renal scans are not necessary after successful pyeloplasty in children, as most operated renal units showed stable renal function for 5-7 years. Further studies are needed to investigate whether renal function changes further after puberty.

Purpose Epididymal anomalies have been reported in 36-79% of undescended testes. In contrast the incidence in normal fetuses is less than 3%. In a post-mortem study in 73 fetuses epididymal anatomy was classified in 6 types (Favorito et al. Eur Urol 1998; 33: 121-3). In this study we investigated the clinical applicability of this classification. Material and Methods During orchidopexy in 82 boys (104 testes) testis position, patency of processus vaginalis and epididymal anatomy was recorded. Eight patients (10%) had a premature birth; in 25 (30%) other congenital anomalies were diagnosed, like Prader Willi syndrome in 3 and Hypospadias in 10. The epididymis was classified as: type 1: the epididymis is connected to the testis at the level of the head and tail; type 2: contact at head, body and tail; type 3: contact at head; type 4: contact at tail; type 5: no contact at head, body and tail; or type 6: partial atresia of epididymis or vas. Type 1 and 2 are considered normal anatomy. Results In 92 (88%) testes the processus vaginalis was patent. The position was abdominal in 29 (28%), canalicular in 39 (37%) and near the external ring in 36 (35%). The epididymal anatomy was: type 1: 37 (36%), type 2: 0 (0%), type 3: 32 (31%), type 4: 15 (14%), type 5: 16 (15%) and type 6: 4 (4%). Anatomy was correlated to the position of the testis: resp. 50%, 33% and 21% of the testes located resp. near the external ring, canalicular and abdominal showed normal contact (type 1). Conclusions Classification of epididymal anatomy in 6 subtypes is applicable in routine clinical practice. Considering subtypes 1 and 2 as normal the prevalence of epididymal anomalies in our series is 64%. Especially subtypes 4, 5 and 6 (in this series 33%) may contribute to impaired fertility. Long-term follow-up studies may provide insight in the clinical significance of epididymal anomalies discovered during orchidopexy in childhood.

PURPOSE Progressive loss of bladder function in children with MMC is an ongoing concern. Urodynamic studies can monitor bladder function changes and the effect of treatment but are available at (half)-yearly intervals at best. We present the pilot data of a non-invasive method to measure bladder pressure at home. MATERIAL AND METHODS During catheterization an extra tube is connected to the catheter. By placing this tube along a vertical ruler the bladder pressure at that time point is measured in cm water and bladder volume (ml) is noted. During regular videourodynamic investigations this method was compared to the standard methodology in 22 patients. For a pilot clinical study we included 5 children with MMC, aged 0-3 years, who were on CIC. Bladder pressure was measured at home during the first morning catheterization by the parents, daily for the first two weeks and twice weekly thereafter. RESULTS The home bladder pressures deviated CONCLUSIONS The home bladder pressure equipment allows reliable measurement of the bladder pressure by the parents. The high frequency allows feedback to the physician on the response of bladder pressure to (changes in) treatment and thereby facilitates fine-tuning of the treatment (medication dose or CIC frequency). In addition, weekly feedback to the parents about the effects of bladder therapy on the bladder pressures of their child increases compliance to therapy.

Purpose Atrasentan (AT) is a highly selective endothelin-receptor-A (ET-A) antagonist. Previously we have shown that the ET-A receptor antagonist LU 302146 (LU) is able to suppress bladder contractions in an acute mini pig model (electrostimulation-induced bladder contractions) The objective of this trial was to evaluate the influence of AT on bladder function and ischemia related changes in bladder structure in a Guinea pig model of urethral obstruction. Material and methods Seven animals were obstructed and received 5 mg/kg/day of Atrasentan for 7 weeks thereafter. Reference groups consisted of obstructed animals or of sham operated animals. Each animal is followed longitudinally with weekly urodynamic investigations. The Atrasentan treated animals were sacrificed at week 8. Details on the experimental set-up are given in [Kok et al. J Urol 2000] Results Compared to the sham-operated group the bladder pressure at maximum flow rate (max. pdet) increased slightly, not significantly, in the AT group and contractility increased significantly. Compared to the obstructed group max pdet was significant lower and contractility similar. The compliance remained normal. Instabilities were not prevented. Structural analysis is currently being performed and will be presented at the ESPU meeting. Conclusions The finding that Atrasentan increases bladder contractility while at the same time maintaining normal bladder compliance suggests that it prevents the anaerobic conditions in the bladder muscle that otherwise lead to hypertrophic and fibrotic changes in the bladder wall. The ongoing structural analysis must show if this is supported by decreased glycogen deposition and whether the preservation of a normal compliance is related to less fibrosis and/or muscle hypertrophy. AT was not as effective in suppressing bladder contractions as LU. The reason might be the different experimental set-up or difference in selectivity of the 2 substances. Future experiments will evaluate the effect of ET-B antagonists on bladder function and structure in the Guinea pig model of urethral obstruction.

Purpose We have previously shown in our prospective study in 42 infants with distal hypospadias that the flow rates both before and after hypospadias repair were impaired when compared to age-matched controls. Aim of present study was to evaluate the long-term flow rates. Material and Methods Thirty/42 boys with distal hypospadias were traced; 21 (mean age 80, range 73-93 months) agreed to participate, resulting in 21 flow curves (rotating disk flowmeter) obtained 59-73 (mean 67) months after the operation. Results Eighteen/21 boys were asymptomatic; 1 showed nocturnal enuresis; 1 nocturnal enuresis with day-time symptoms; and 1 incontinent boy was dry with anticholinergic medication. 38% of the long-term flows (vs. 50 % of the short term flows) were intermittent. The Qmax averaged 13,2 ± 5,7 ml/s. In comparison, the short-term (3 and 9 months postop) Qmax were 6,6 ± 2,8 and 7,2 ± 1,8 ml/s. The majority (71%) of the flows was low-normal (5th to 50th percentile); 10% was above the 50th and 19% was below the 5th percentile. In the short term 76% of the flows was below the 5th percentile. Conclusions In this prospective study short-term (3-9 months postoperative) flow rates decreased, but long-term (5.5 years) flow rates improved to low-normal values. We hypothesize that the low flow rates after hypospadias repair are caused by a reduced compliance of the distal urethra, lacking a surrounding spongiosal body. Improvement of the long-term vs. short-term flow rates might be explained by decline of postoperative oedema and increase in urethral diameter with growth of the child. We suggest long-term follow-up with uroflowmetry in asymptomatic boys after hypospadias correction. Only in case of symptoms, persistently too low (

Purpose In an animal model of urethral obstruction glycogen deposition in the bladder muscle was found to be a marker for previous increased voiding pressure and decreased compliance [de Jong et al. Neurourol Urodynam 2008; 27: 454-60]. Here we measured if also in the human bladder glycogen deposition marks adaptation to increased pressure in the filling phase by comparing two groups: normal leak-point pressure + normal compliance (Vesico Ureteral Reflux) and high leak-point pressure + low compliance (MyeloMeningoCele). Material and Methods Rest material was obtained from patients undergoing a ureter reimplantation (VUR group, n = 8) or an augmentation (MMC group, n = 16). Glycogen deposition was scored from 0 to 3 on periodate acid Schiffs (PAS) stained sections. Bladder function was assessed with video urodynamics yielding: CAPacity (ml), COMpliance (from 0,good, to 2, bad), INStability (0,absent, to 2, severe), Leak Point Pressure (cm water). Significance of difference was tested with a student t-test. Results Group PAS Cap COM INS LPP VVR group 1.6 ± 0.3 217 ± 47 0.29 ± 0.17 0.13 ± 0.13 17 ± 3 MMC group 2.5 ± 0.12 83 ± 11 1.19 ± 0.15 0.8 ± 0.23 64 ± 5 p -value Discussion In Guinea pigs glycogen deposition in the bladder muscle marks adaptation to prolonged periods of increased bladder pressure. This marker function has now been confirmed for the human bladder. As glycogen is deposited in response to ischemia this suggests the increased pressure is linked with ischemic conditions. Next to extrinsic conditions like an obstruction downstream this intrinsic bladder condition might be involved in the continuing loss of bladder function that, often unexplained, occurs in patients with structural/functional obstruction. This opens new venues for monitoring bladder function (bladder wall blood supply and saturation) and new angles of treatment (maintaining proper bladderwall saturation).

Purpose Although Frank already described vaginal dilation therapy for treatment of a short vagina in 1938 patient selection criteria, treatment protocols and outcome parameters are still poorly defined. Since 2000 vaginal dilation is our first-line treatment in 46 XY-DSD with female gender. We present data of 15 consecutive patients. Material and Methods In 15 adolescents (mean age 16 years) vaginal length was measured under anesthesia and the compliance of the vaginal vault was assessed. Diagnosis was Androgen Insensitivity Syndrome in 9 (group A) and other cause, like T-synthesis disorder, in 6 (group B). In 5 patients from group A the vagina was considered sufficient. Four/9 from group A and all 6 from group B were considered candidates for selfdilation therapy. After psychological counseling and instruction patients were advised to dilate the vagina daily with a Hegar sound during 3-5 minutes. Results Mean vaginal length in group A was 5 (range 3-10) cm and in group B 3 (range 2-5) cm. Mean vaginal length in the 5/15 who did not require therapy was 7 (range 5-10) cm. Mean vaginal length in the therapy group was 3 (range 2-5) cm. Three/10 completed dilation therapy after 12-24 months with an average increase in vaginal length of 5,7 cm; 1/10 discontinued selfdilation after successful coitus. Three/10 are not yet ready for treatment; 3/10 are lost to follow-up. Conclusions Although follow-up is short and information on sexual function is lacking in most patients to allow definitive conclusions we consider vaginal self-dilation therapy as treatment of first choice in 46 XY-DSD patients with a short vagina. Patient motivation and a delicate approach in a multidisciplinary setting are keystones to a successful result of dilation treatment.